Post-polio syndrome

Management
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By Tarakad S Ramachandran MD and Arun Ramachandran

Generalized fatigue is best treated with lifestyle changes consisting of energy conservation measures (frequent rest periods and daytime naps, pacing of physical activities), weight-loss programs, and the use of assistive devices (orthoses, canes, intermittent use of wheelchairs) (Waring et al 1989; Peach and Olejnik 1991). Pharmacological agents (amantadine hydrochloride, pyridostigmine bromide, fluoxetine hydrochloride, pemoline, Ritalin, modafinil) may have a role in the amelioration of generalized fatigue, but only 3 of these agents (amantadine and pyridostigmine, and modafinil) have been studied in a controlled fashion, and all 3 were found to lack benefit (Dalakas 1999; Trojan et al 1999; Chan et al 2006).

The most important advance in the treatment of new post-polio syndrome weakness is the finding in at least 9 studies that mild-to-moderate weakness can be improved with a nonfatiguing exercise program (Ernstoff et al 1996; Agre et al 1997; Jubelt and Drucker 1999; Chan et al 2003). All of these studies demonstrated increased muscle strength and none showed laboratory evidence of muscle overuse (eg, increase in creatinine kinase or electromyographic or biopsy evidence of muscle damage). These nonfatiguing exercise programs avoid overuse by using both submaximal and maximal strength combined with short-duration repetitions.

Exercise should be performed on alternate days to allow for full recovery and to avoid overuse. A physical therapist is needed to instruct patients on appropriate exercise techniques and to monitor the patients so they do not overexert. Patients with post-polio syndrome who were able to exercise at a level that avoided overuse (excessive muscle fatigue or increasing muscle or joint pain) have experienced positive results (Agre and Rodriquez 1997). Muscle fatigue, can also be improved by interspersing bouts of activity with rest periods (pacing) to avoid excessive fatigue (Agre and Rodriquez 1991). This simple procedure significantly improved strength recovery after activity (Agre and Rodriquez 1991). Patients with post-polio syndrome who exert their weak muscles to the point of exhaustion (overuse) may require 2 to 3 days to recover from the resulting muscle fatigue. Pharmacological intervention with pyridostigmine and prednisone did not improve muscle strength (Dalakas 1999; Trojan et al 1999).

Bulbar muscle weakness may also lead to dysphagia, respiratory failure and sleep disorders. Dysphagia can be improved by instruction on swallowing techniques (Silbergleit et al 1991). Chronic respiratory failure can often be managed with nighttime noninvasive positive-pressure ventilation (Bach 1995). Mouth and nasal intermittent positive pressure ventilation, manually and mechanically assisted coughing, and noninvasive blood gas monitoring in the home were the main techniques used for optimizing quality of life and for avoiding complications (Bach 1987b). With the use of these measures, acute respiratory failure and tracheal intubation can usually be avoided (Bach 1995). Only a small percentage of patients eventually require tracheotomy and permanent ventilation. Although not evidenced based, influenzal and pneumonococcal vaccines are probably indicated. Smoking should be eliminated and obstructive disease treated. Sleep disorders occur frequently in post-polio syndrome patients and are treated similarly to sleep apnea seen in patients without post-polio syndrome (Jubelt and Drucker 1999). In patients with post-polio syndrome, Hsu and Staats identified 3 patterns of sleep disturbances: (1) obstructive sleep apnea, (2) hypoventilation, and (3) a combination of both (Hsu and Staats 1998). They further proposed that sleep-related disordered breathing (SRDB) is a late sequela of poliomyelitis, and clinical evaluation should include information about sleep. Another study found that transcranial direct current stimulation (tDCS) over the premotor areas for 15 days improved sleep and fatigue symptoms in patients with post-polio syndrome (Acler et al 2013). Acler and colleagues further added that tDCS could be a noninvasive and valuable new tool for managing post-polio patients.

Musculoskeletal pain (muscle and joint pain) and joint instability can be treated with braces and wheelchairs (Waring et al 1989), pacing activities (Willen and Grimby 1998) and lifestyle changes (Peach and Olejnik 1991). Anti-inflammatory medications, anti-depressant pain medications (eg, amitriptyline, duloxetine), heat, and massage appear to be somewhat beneficial but have not been studied objectively. In a small, randomized controlled trial, lamotrigine significantly improved pain, muscle cramps, and fatigue at 2 and 4 weeks (On et al 2005).

Cardiac conditioning is important for all patients, and three trials using aerobic or general conditioning exercise with lower-limb cycle or arm ergometers have shown significant improvement in cardiorespiratory fitness in post-polio syndrome patients (Kriz et al 1992; Ernstoff et al 1996; Agre and Rodriquez 1997). Two aquatic exercise studies have demonstrated that flexibility, strength, and cardiorespiratory fitness can be improved in post-polio syndrome patients (Prins et al 1994; Willen 1999). Finally, it is important to note that psychological symptoms related to the reemergence of a supposedly resolved disease and to the stresses of the needed major lifestyle changes can be overwhelming at times (Jubelt and Drucker 1999; Kemp and Krause 1999).

Table 2. Evidence-Based Treatment for Patients with Post-polio Syndrome*†

Generalized fatigue

  • Institute lifestyle changes including energy conservation and weight-loss programs
  • Prescribe appropriate lower extremity orthoses

Muscle weakness and fatigue

  • Institute a nonfatiguing strengthening exercise program
  • Institute physical activity pacing with rest periods
  • Avoid overuse of weakened muscles

Bulbar muscle weakness

  • Dysphagia
    - Instruction on swallowing techniques
  • Respiratory failure
    - Noninvasive positive-pressure ventilation at night and as needed
    - Tracheostomy and permanent ventilation

Musculoskeletal pain and joint instability

  • Decrease mechanical stress on joints and muscles
    - Lifestyle changes such as weight loss, pacing activities,
    - Using assistive devices (including orthoses, wheelchairs, and adaptive equipment)
  • Prescribe antiinflammatory medications, anti-depressant pain medications, lamotrigine, heat, massage

Cardiopulmonary conditioning

  • Cycle or arm ergometer exercise
  • Aquatic exercise training

*All of the interventions are evidence-based except for tracheostomy and permanent ventilation; and prescribing antiinflammatory medications, anti-depressant pain medications, heat and massage.

†Adapted from (Jubelt and Agre 2000). See text for references.

Future randomized trials should also address the long-term effects of muscular training in post-polio syndrome in addition to treatment of pain in these patients. In their randomized controlled clinical trial, based on the utilized measures of outcome, Vasconcelos and colleagues found that modafinil was not superior to placebo in alleviating fatigue or improving quality of life in the studied post-polio syndrome population (Vasconcelos et al 2007). Farbu and colleagues, in their small pilot study on post-polio syndrome patients, concluded that no effect was seen with intravenous immunoglobulin treatment on muscle strength and fatigue; however, intravenous immunoglobulin-treated post-polio syndrome patients reported significantly less pain 3 months after treatment (Farbu et al 2007). TNF-alpha was increased in the CSF of post-polio syndrome patients.

Willen and colleagues found only minor changes in disability during a 4-year period of follow-up in post-polio patients (Willen et al 2007). It is hoped that support from the polio clinic may result in self-selected lifestyle changes, which may positively influence the development of symptoms and functional capacity. Wise reported that patient education, health promotion, and energy conservation strategies, which included walking with a properly fitting assistive device, reduced perceived fatigue and improved posture and function in an individual with post-polio syndrome (Wise 2006).

In their report of an EFNS task force on post-polio syndrome, Farbu and colleagues recommend Halstead's definition of post-polio syndrome from 1991 as diagnostic criteria (Farbu et al 2006). They feel supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness, and pain. Farbu and colleagues note that training in a warm climate and non-swimming water exercises are particularly useful (Level B). They further add that recognition of respiratory impairment and early introduction of noninvasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C).

Based on their review of the literature on post-polio syndrome, Aguila-Maturana and Alegre-De Miquel conclude that lamotrigine, bromocriptine, aerobics and flexibility exercises, hydrokinesitherapy, and technical aids are treatment techniques that reduce fatigue more in these patients (Aguila-Maturana and Alegre-De Miquel 2010).

Randomized control studies had shown that intravenous immunoglobulin is effective to reduce pain in complex regional pain syndrome (low-dose intravenous immunoglobulin) and post-polio syndrome (high-dose intravenous immunoglobulin) (Goebel 2010). Open trials have also shown efficacy in additional pain conditions. But questions still linger around the optimal treatment doses, duration of treatment, and its effect on function and quality of life.

Dysphagia and dysphonia occurring with post-polio syndrome often pose a challenge with neuro-rehabilitation in these patients (Söderholm et al 2010). Routine evaluation of dysphagia and dysphonia must be carried out in all patients with post-polio syndrome.

Osteopenia and osteoporosis are increasingly associated with post-polio syndrome, predisposing to increased fracture risk (Mohammad et al 2009). Thus, bone density assessment, review of falls risk, and therapeutic intervention should be considered for all patients.

Expression of inflammatory cytokines in cerebrospinal fluid (CSF) has led to the hypothesis of intrathecal chronic inflammation to explain the denervation observed in post-polio syndrome (Gonzalez et al 2002; Fordyce et al 2008). Ostlund and associates found that IVIG leads to an increase of quality of life at 6-month follow-up by improving vitality, social function, and role emotional, with significant decrease in bodily pain (Ostlund et al 2012). Gonzalez and colleagues have further shown that IVIG therapy has sustained positive effects on relevant QoL variables and inflammatory cytokines up to 1 year in patients with post-polio syndrome (Gonzalez et al 2012).

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Etiology
Pathogenesis and pathophysiology
Epidemiology
Prevention
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
Anesthesia
References cited
Contributors